AIM: To study PF-ILD among HSP patients (pts).

Method: Pts diagnosed at ILD multidisciplinary meeting from Jan 1, 2014 to Dec 31, 2020 with a minimum 24-mnth follow-up (FU) period were included. Pts demographic, laboratory data & CT chest grading (1=non-fibrotic, 2=reticulation & traction bronchiectasis, 3=honeycombing) were analysed. Disease progression was a relative forced vital capacity (FVC) decline ?10%, death, lung transplantation or any two of: relative FVC decline ?5% & <10%, progressive symptoms or fibrosis on CT within 24 mnths of diagnosis. 2-sample t test, Binary logistic regression (BLR), Cox Hazards Model & Kaplan?Meier analysis were used.

Result: 56 pts were included. Mean age±sd 66.5±13.1 yrs. 33 (59%) were females. 25 (45%) were smoker/exsmoker (mean PYES±sd 19.6±14.6). Antigen exposure was identified in 28 (50%). PF-ILD occurred in 26 (46.4%) pts. The PF-ILD group (grp) had a higher BMI (p<0.05) & ?2 comorbidities (p<0.05) vs NonPF-ILD grp. There was no difference in the exposure history, baseline lung function & CT grading. On BLR only BMI was a risk for progression (odds ratio,1.100;95% confidence interval,1.000-1.220;p<0.05).

36 (64%) pts were alive at study conclusion. Median FU was 54-mnths (95%CI;45-76 mnths). The 5-yrs survival was 59% for non-PFILD grp vs 51% in PF-ILD grp (p<0.05). In the PF-ILD grp, age>65 yrs, FEV1<65% predicted, TLC<65% predicted, a higher CT grading & progression <12 mnths of diagnosis predicted (p<0.05) worse survival.

Conclusion: Nearly half of the pts with PF-ILD died within 5 years. Baseline laboratory & CT changes did not identify the at risk grp. Regular FU & early escalation of therapy is essential to improve outcomes.